Childhood CNS inflammatory demyelinating diseases

CNS inflammatory demyelinating diseases are rare disorders in childhood and at first presentation children are diagnosed with acute disseminated encephalomyelitis (ADEM), clinically isolated syndrome (CIS) such as transverse myelitis, or Neuromyelitis Optica. These diseases may culminate in physical and cognitive disability or ultimately be diagnosed as Multiple Sclerosis (MS). MS is a chronic inflammatory neurodegenerative demyelinating disease of the CNS that is usually relapsing remitting at onset. There has been significant recent interest and progress in these disorders culminating in the creation of consensus definitions by the International Paediatric Multiple Sclerosis Study Group (2007). Encephalopathy (behavioural change or altered consciousness) distinguishes ADEM from other demyelinating conditions. A high index of suspicion for CNS inflammatory demyelinating diseases is required in children presenting with neurological deficits, encephalopathy, and first onset status epilepticus. Several UK and international studies are underway to further our understanding of these diseases. In this review we describe current understanding of the epidemiology, pathogenesis, clinical features, outcome and management of childhood CNS inflammatory demyelinating diseases.